Endocrine Abstracts

Section 1: Case history : The case concerns a 53-year-old gentleman who developed calciphylaxis following a bioprosthetic aortic valve replacement. His past medical history included Multiple Endocrine Neoplasia Type 1 (MEN-1) for which he had previously undergone total parathyroidectomy with failed graft auto-transplantation. The resulting hypoparathyroidism was managed with alfacalcidol and calcium supplements. Post cardiac valve replacement he developed acute renal injury and necrotic lesions started to appear on the skin of his buttocks, abdomen and limbs.

Section 2: Investigations : An incisional skin biopsy confirmed the histological diagnosis of calciphylaxis with Von Kossa staining demonstrating widespread calcium deposition in vessel walls and within the subcutis. At time of diagnosis, adjusted calcium was 3.03mmol/l and phosphate level 1.88mmol/l, in-keeping with overtreatment of hypoparathyroidism.

Section 3: Results and treatment : The calciphylaxis was managed conservatively with regular wound care and aggressive antibiotic treatment for infections in order to prevent death from sepsis. To reduce further ex-ossesous soft tissue calcification his calcium and phosphate levels were carefully managed with titration of alfacalcidol and calcium supplements, including the phosphate binder calcium acetate. During his 10 month inpatient stay he developed other complications including chest infection, acute kidney injury and wound haemorrhage. He was bed bound for the majority of his time in hospital which triggered immobility hypercalcaemia. This was treated with infusions of pamidronate and total cessation of alfacalcidol and calcium supplements. He required extensive therapy input and was even supported in achieving a personal goal of attending an international football match with his teenage daughter. Despite many setbacks, the patient survived and was discharged home where he has now regained his mobility and remains under close monitoring.

Section 4: Conclusions and points for discussion : Calciphylaxis is a rare live threatening disorder characterised by painful necrotic lesions of the skin. Ex-osseous soft tissue calcification leading to occlusion of cutaneous arterioles resulting in tissue necrosis. It has an exceptionally high mortality – up to 80% - with most deaths occurring due to sepsis. The vast majority of cases occur in patients with chronic renal disease with only a handful ever reported in the context of hypoparathyroidism. Management of calciphylaxis centres on controlling calcium and phosphate levels, wound care, pain management and prevention of sepsis. In conclusion, this case describes an often-fatal condition occurring in unusual circumstances and the management that led to the patient’s survival against the odds!